获得性大疱性表皮松解症1例

获得性大疱性表皮松解症（epiderrnolysis bullosa acquisita EBA)是一种自身免疫性表皮下大疱病，以摩擦或外伤部位出现张力性水疱及血清内存在高滴度胶原自身抗体为基本特征，本病临床较少见，且临床表现和其他大疱性皮肤病类似，较易引起误诊，现将本科诊治的1例报告如下。     

获得性大疱性表皮松解症3例报告

获得性大疱性表皮松解症３例报告周曙霞，张学军，翁孟武，施守义上海医科大学华山医院皮肤科（邮政编码２０００４０）获得性大疱性表皮松解症（ＥＢＡ）是一种较少见的自身免疫性大疱性皮肤病．皮肤受轻微外伤或摩擦后即起水疱甚至大疱，愈后留萎缩性瘢痕及粟丘疹，同时...     

获得性大疱性表皮松解症

报告1例获得性大疱性表皮松解症.患者男,56岁.全身摩擦部位皮肤经摩擦后起大疱2年余.2年多以来,躯体受摩擦部位易擦伤、起大疱和结痂,无疼痛和瘙痒,病情反复,且伴有白色丘疹.体格检查可见躯干和四肢有大片红斑、糜烂、水疱和结痂,尤以易摩擦部位为重,双手和耳郭可见白色丘疹,甲变形、断裂.皮损组织病理检查示表皮下大疱和粟丘疹,直接免疫荧光检查在基膜下可见IgG呈线状沉积.根据典型的临床表现、组织病理改变和免疫荧光的检查结果诊断为获得性大疱性表皮松解症.给予糖皮质激素口服治疗.     

获得性大疱性表皮松解症伴肝硬化腹水死亡1例

患者,男,５４岁,因双手足、四肢伸侧、骶部散在水疱、渗液、结痂、疤痕１３年伴纳差、腹胀８月余入院。患者自１９８４年１２月份因外伤左手腕部出现黄豆大水疱,疱破后糜烂、渗液,几日后自行结痂,渐肘、膝、骶部等易摩擦处均出现水疱,疱易破,渗液粘稠,结痂处愈合后遗留萎缩性疤痕。曾在上海华山医院病理检查后诊为“获得性大疱性表皮松解症”,间断服用过强的松３０—４０ｍｇ／日,以及支持治疗（白蛋白、能量合剂、各种维生素）,病情时轻时重。近８月来突觉纳差、腹胀,偶伴恶心、呕吐,呕吐为非喷射性,同时伴吞咽困难。并出现…     

获得性大疱性表皮松解症的研究进展

获得性大疱性表皮松解症是一种自身免疫性慢性大疱性皮肤病,临床可分为两种类型:经典型和炎症型,血循环中有抗Ⅶ型胶原的自身抗体。诊断获得性大疱性表皮松解症除根据临床表现、组织病理、直接和间接免疫荧光外,还需做一些特殊检查,包括盐裂免疫荧光、免疫印迹、免疫电镜和ELISA检查。需要与其他自身免疫性表皮下疱病相鉴别。治疗主要是系统应用糖皮质激素、免疫抑制剂和抗炎药物。     

儿童获得性大疱性表皮松解症一例

报道1例儿童获得性大疱性表皮松解症.患者女,12岁,因全身反复水疱、大疱和糜烂面3个月入院.患者弱智,父母非近亲结婚,无家族史.入院时表现全身大片糜烂面,包括手足、膝关节周围和股臀部,同时躯干、四肢正常或水肿性红斑基础上紧张性水疱、大疱.治疗过程中突然发生躯干、四肢大量紧张性水疱.组织病理提示,表皮下水疱,免疫荧光结果见表皮基底膜带IgG和C3线状沉积,盐裂后沉积限于真皮侧.ELISA检测BP180和BP230阴性.采用大剂量糖皮质激素联合四环素口服取得满意疗效.

Abstract：

A case of epidermolysis bullosa acquisita (EBA) in childhood is reported. A 12-year-old girl was hospitalized for a 3-month history of recurrent blisters, bullae and erosions on the trunk and limbs. The girl had mental retardation but no family history of similar disorders. The marriage between her parents was not consanguineous. Physical examination on admission revealed large erosions with moderate oozing on the hands and feet, around the knees and on the buttock and thighs. There were scattered tense blisters and bullae arising in normal skin or edematous erythema on the trunk and limbs. During the treatment course the patient suddenly developed a number of tense blisters over the whole integument on the trunk and limbs. Skin biopsy showed subepidermal bullae with moderate perivascular infiltration of neutrophils and eosinophils. Direct immunofluorescence (DIF) revealed linear IgG and C3 deposition along the basal membrane zone, which was on the dermal side of salt-split skin on indirect immunofluorescence (IIF). ELISA detected no serum antiBP180 or -BP230 antibodies in the patient. A diagnosis of EBA was made. The patient was successfully controlled by intravenous steroids combined with oral tetracycline.     

获得性大疱性表皮松解症误诊一例并文献复习

报道一例反复误诊的获得性大疱表皮松解症并对相关文献进行复习。患者,女,25岁。皮疹泛发全身,主要表现为张力性水疱,疱壁紧张,尼氏征阴性,在外院误诊为天疱疮、线状IgA大疱病。组织病理检查示:表皮下水疱;盐裂IIF：IgG沉积在真皮侧; DIF:表皮基底膜IgG、C3、IgM、IgA带状沉积,ELISA：BP180,BP230均阴性,诊断为获得性大疱性表皮松解症,给予甲泼尼龙、氨苯砜、人免疫球蛋白、吗替麦考酚酯等治疗,病情好转。     

Case Report : Epidermolysis Bullosa Acquisita

An example of epidermolysis bullosa acquisita arising in a middle-aged man is presented. The theory of basement membrane disruption is supported. No new light is shed but possibly a useful form of treatment was found.     

Premonitory Epidermolysis Bullosa Acquisita Mimicking Eyelid Dermatitis

We report a unique case of a 71-year-old female patient with epidermolysis bullosa acquisita. The patient initially presented with the clinical symptoms of bilateral eyelid dermatitis that occurred several months prior to the development of oral and pharyngeal erosions and blisters. While no contact allergy was found by patch testing, direct immunofluorescence microscopy demonstrated linear deposits of IgG at the basement membrane zone. By indirect immunofluorescence microscopy on human salt-split skin, IgG antibodies bound to the dermal side of the split. Immunoblot analysis showed predominant IgG4 reactivity of the patient’s serum with the recombinant non-collagenous-1 domain of type VII collagen. Because of treatment resistance to systemic corticosteroids, dapsone, and colchicine, we initiated a combination treatment of protein A immunoapheresis and rituximab. With this treatment, complete remission was achieved within 4 months. Our case highlights that epidermolysis bullosa acquisita may initially mimic an eyelid dermatitis. Consequently, dermatologists should be aware of this rare differential diagnosis of eyelid dermatitis where a contact allergy or atopic dermatitis is absent.     

Epidermolysis Bullosa Acquisita Localized to the Face

A 39-year-old woman had a three-year history of recurrent bullous eruption localized to her left cheeck. The diagnosis of epidermolysis bullosa acquisita was confirmed by means of direct immunofluorescence and direct immunoelectron microscopic studies performed on the perilesional salt-split skin. Topical corticosteroid treatment reduced pruritus and bullae formation. This case of localized epidermolysis bullosa acquisita on the face is reminiscent of Brunsting-Perry cicatricial pemphigoid. We also review the previously reported cases of localized epidermolysis bullosa acquisita.     

Epidermolysis Bullosa Acquisita Responsive to Dapsone Therapy

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a chronic subepidermal blistering disease that is frequently resistant to therapy. OBJECTIVE: A 58-year-old man who had a one-year history of a bullous eruption involving the hands, forearms, trunk, scalp, and oral mucosa. Histopathology revealed a subepidermal bulla, and direct and indirect immunofluorescence studies were consistent with EBA. The patient failed respond to niacinamide and tetracycline and oral prednisone 40 mg per day. METHODS: Complete control of his blistering was achieved within two months of initiating oral dapsone, 150 mg per day. CONCLUSION: Dapsone may be an effective agent for some patients with EBA.     

Epidermolysis Bullosa Acquisita in an 8-year-old Girl

Epidermolysis bullosa acquisita is an autoimmune blistering disease with the distinct feature of having an autoantibody directed against an antigen located below the basement membrane of human skin and mucous membrane. We identified this disease in an 8-year-old girl, the youngest patient documented by immunoelectron microscopy.     

A Case of Recalcitrant Epidermolysis Bullosa Acquisita Responsive to Rituximab Therapy

Epidermolysis bullosa acquisita (EBA) is an autoimmune blistering disorder that occurs infrequently in children. Although typically associated with a good prognosis in children, there are rare cases that are refractory to treatment with conventional immunosuppressive therapy. Rituximab, an anti‐CD20 monoclonal antibody, has been reported to be effective in the adult form of EBA. We report a case of a child with severe and recalcitrant EBA exhibiting a sustained response to treatment with rituximab.     

Epidermolysis Bullosa Acquisita (EBA) with Nonclassical Distribution of Eruptions

The skin lesions in epidermolysis bullosa acquisita (EBA), a mechanobullous disease, often show acral distribution. Recently, we experienced a case of EBA in which most of the skin lesions were located on the trunk. We reviewed the distribution of the skin eruptions in 58 reported cases of EBA. Although the extensor surfaces of the extremities are the most common site, there were some cases with non-acral distribution. These “nonclassical” cases should also be considered in the clinical diagnosis of EBA and other bullous diseases.     

Linear IgA Disease with Clinical and Immunopathological Features of Epidermolysis Bullosa Acquisita

Abstract: 10-year-old boy had a 3-month history of urticarial plaques and vesicles. Histologic and immunofluorescence testing confirmed the diagnosis of linear IgA disease. Immunoelectron microscopy revealed IgA deposits in the sublamina densa area similar to those seen in epidermolysis bullosa acquisita. Milia developed after resolution of the lesions, similar to lesions of epidermolysis bullosa acquisita.